Abstract
Absence of the right superior vena cava with persistent left superior vena cava without any other cardiac anomalies in visceroatrial situs solitus is rare. A 41-year-old man presented with a feeling of anterior chest pressure on effort. Severe mitral regurgitation due to anterior prolapse of the A2-A3 segments and moderate tricuspid regurgitation were diagnosed. Three-dimensional CT scan revealed an absent right and persistent left superior vena cava. Electrocardiographic findings showed a typical coronary sinus rhythm. At operation, a pulmonary artery catheter was placed via the right femoral vein under fluoroscopy. After starting cardiopulmonary bypass with a single venous cannula in the inferior vena cava, an L-shaped venous cannula was directly placed into the left superior vena cava. Mitral valve repair was performed, with 4 pairs of Gore-Tex CV-5 artificial chordae and mitral ring annuloplasty through a standard transverse left atriotomy. Tricuspid ring annuloplasty was also performed. His postoperative course was uneventful. Postoperative echocardiography showed only trivial mitral and tricuspid regurgitation. In patients with such venous anomalies, the area around the coronary sinus should be protected during intracardiac procedures to preserve the coronary sinus rhythm. We therefore recommend direct venous cannulation of the left superior vena cava instead of retrograde cannulation via the coronary sinus, and standard transverse left atriotomy for mitral exposure.
